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J Thorac Cardiovasc Surg 1994;107:1272-1283
© 1994 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Tokyo, Japan
Address for reprints: Yasuharu Imai, MD, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawadacho, Shinjuku-ku, Tokyo 162, Japan.
Abstract
Since June 1989, 18 patients with atrioventricular discordant anomalies under 15 years of age underwent anatomic correction, or double switch operation, with 2 hospital deaths (11%). Ages ranged from 1 year 4 months to 12 years (mean 7.2 years) and body weights ranged from 7.1 to 32.5 kg (mean 20.1 kg) at operation. Visceral situs was solitus in 12 and inversus in 6. Ventriculoarterial connection was discordant in 4 and double-outlet in 14. All had associated congenital cardiac anomalies consisting of ventricular septal defect in 17, atrial septal defect in 9, pulmonary atresia in 10, pulmonary stenosis in 6, systemic tricuspid regurgitation in 9, mitral regurgitation in 6, bilateral superior venae cavae in 6, patent ductus arteriosus in 6, Wolff-Parkinson-White syndrome in 2, and congenital atrioventricular block in 1. Previous palliation had been performed 19 times in 11 patients including systemic-pulmonary shunts in 10 patients and pulmonary banding in 1 patient. Anatomic correction consisted in the Mustard procedure in 12 or the Senning procedure in 6 at the atrial level and in external conduit repair in 14, arterial switch operations in 3, or direct anastomosis between the pulmonary artery and right ventricle in 1 at the ventriculoarterial level. The arterial switch operation was indicated in patients with a normal pulmonary valve, and external conduit repair was indicated for patients with pulmonic stenosis or atresia. Anatomic right ventricular end-diastolic volume showed significant reduction from 122.2% ± 46.3% of normal to 78.8% ± 24.0%, mainly owing to unloading of the ventricle after the operation (p < 0.0003). Right ventricular ejection fraction remained unchanged (56% ± 8% to 56% ± 6%). Anatomic left ventricular end-diastolic volume and left ventricular ejection fraction remained unchanged before and after the operation: volume 134.6% ± 50.0% of normal to 127.3% ± 32.8% and ejection fraction from 61% ± 8% to 56% ± 8%. Cardiac index averaged 3.1 ± 0.5 L/min per square meter after the operation. The double switch operation is best indicated in patients with atrioventricular discordance associated with systemic tricuspid regurgitation and/or with poor systemic right ventricular function. (J THORAC CARDIOVASC SURG 1994;107:1272-83)
Atrioventricular (AV) discordant anomalies have unique features in the AV as well as ventriculoarterial (VA) arrangements, in which the anatomic right ventricle (RV) serves as a systemic ventricle and the anatomic left ventricle (LV) as a pulmonic counterpart. Although conventional repair yielded excellent results on a short-term basis, long-term systemic RV function is still a matter of concern.
1-6 Deterioration of systemic RV function associated with tricuspid regurgitation in patients with complete transposition of the great arteries after Mustard or Senning procedures 7-14 also suggests poor systemic RV function in AV discordance.
The double switch operation for AV discordant anomalies has the advantage of creating nearly normal AV and VA relationships, in which the anatomic LV serves as a systemic ventricle and the anatomic RV functions as a pulmonic ventricle after the operation. The purpose of this report is to present our series of anatomic repair of AV discordance and to compare its postoperative ventricular function with that of the conventional external conduit repair in AV discordance and in complete transposition of the great arteries.
PATIENTS AND METHODS
From June 1989 to April 1993, 18 patients with AV discordance under 15 years of age underwent the double switch operation, consisting of a Mustard procedure in 12 patients and a Senning procedure in 6 at the atrial level and of an arterial switch operation in 3, an external conduit repair in 14, and direct anastomosis between the pulmonary artery and RV in 1 patient at the VA level. The Mustard procedure with patch augmentation of the functionally systemic atrium was selected for small right atrium, and arterial switch was done in patients with an intact pulmonary valve (Tables I and II).
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Associated anomalies were seen in all cases: VSD in 17, atrial septal defect in 9, pulmonary atresia in 10, pulmonary stenosis in 6, bilateral superior vena cava in 6, patent ductus arteriosus in 6, Wolff-Parkinson-White syndrome in 2, congenital AV block in 1, and single coronary artery in 1. Systemic tricuspid regurgitation was seen in 9, mitral regurgitation in 6, and aortic regurgitation in 4 patients. Situs solitus {SDD} was seen in 12 and situs inversus {ILL} in 6 patients. Risk factors regarding application of conventional repair by using the systemic RV were observed in 15 of 18 patients (83%), including tricuspid regurgitation, poor ejection fraction, small RV volume, or large RV volume (Table III).
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15 Volume occupied by papillary muscles for the LV was adjusted according to the method of Graham, Jarmakani, and Canent 16 as follows: When the measured LV volume (V) was less than 15 ml, adjusted LV volume was measured as follows: V' = 0.874 V – 3.1. For LV volume more than 15 ml, the following equation was applied: V' = 0.733V. Similarly, volume for the morphologically RV, calculated by Simpson's method, was adjusted by the following formula: V' = 0.649V. Both adjusted ventricular volumes were then expressed as a percentage of the expected normal volume of the respective ventricle per body surface area (BSA) according to the formula by Nakazawa and associates 17 as follows:Postoperatively, pulmonic ventricular volume and its ejection fraction were calculated by excluding the subvalvular portion of the external conduit in all types of external conduit repair both in conventional repair and in the double switch operation. Regarding stroke volume index, commonly expressed as milliliters per square meter of body surface area, ventricular end-diastolic volume (expressed as percent of normal value) multiplied by ejection fraction was arbitrarily termed as percent stroke volume index in this article for the sake of standardization and of easy comparison among patients with different body surface areas.
All mean values were expressed as a mean ± 1 standard deviation, and the paired or unpaired t test was used for statistical analysis.
Preoperative RV and LV pressures were equal in all patients; cardiothoracic ratio averaged 50.9% ± 6.4%, ranging from 40% to 63%; pulmonary artery index 18 ranged from 182 to 593 (mean 332.3 ± 127.5 mm2/BSA); systemic arterial partial pressure of oxygen ranged from 30 to 84 mm Hg with a mean of 43.6 ± 13.2 mm Hg; systemic arterial oxygen saturation ranged from 56% to 97% (mean 60.9% ± 7.8%); and pulmonary/systemic flow ratio ranged from 0.33 to 2.24 (mean 1.1 ± 0.6). Preoperative systemic RV end-diastolic volume ranged from 62% to 241%, with a mean of 122.2% ± 43.6%; RV ejection fraction ranged from 41% to 73% (mean 56% ± 8%); pulmonic LV end-diastolic volume ranged from 82% to 269%, averaging 134.6% ± 50.0%; and LV ejection fraction ranged from 40% to 75% (mean 61% ± 8%).
Postoperative catheterization and angiography were performed in 15 patients from 18 to 67 days (mean 32.4 ± 12.9 days) after operation and in the other 2 patients 1
years later. Systemic ventricular function after the double switch operation was compared with that after conventional external conduit repair, bridging between the LV apex and pulmonary artery in 20 patients with AV discordance in our series. Also, ventricular function after the double switch operation, consisting of external conduit repair combined with the Mustard or Senning procedure, was compared with that in 23 patients with complete transposition with VSD and with reduced pulmonary blood flow who underwent external conduit repair approximately in the same period in our institution. Catheterization was performed 22 to 56 days (mean 30.8 ± 8.5 days) after the operation in patients with AV discordance who underwent conventional conduit repair and 22 to 95 days (mean 38.1 ± 19.6 days) after the operation in patients with transposition who underwent conduit repair.
Operative techniques
Intracardiac repair was performed through a median sternotomy with the use of moderately hypothermic cardiopulmonary bypass (25° to 27° C) via ascending aortic and direct bicaval or tricaval cannulation. Glucose-insulin-potassium solution (20 ml/kg) was used to induce cardioplegia and was repeated in half the dose every 40 minutes thereafter. The cardioplegic solution contained 20 mEq of potassium and 20 units of regular insulin in a liter of 5% dextrose solution. As an atrial switch procedure, the Senning repair was preferred in patients with a normal or large right atrium, and the Mustard procedure with liberal patch augmentation of the functional left atrium was chosen for patients with a small right atrium because of an overhanging anatomic LV in mesocardia or dextrocardia in situs solitus. At the VA level, an arterial switch operation was performed in patients with a normal pulmonary valve, and external conduit repair with intraventricular rerouting was preferred for those with pulmonary atresia or pulmonary valvular stenosis. In patient 18, RV outflow reconstruction with a monocusp patch was performed after direct anastomosis between the RV and distal pulmonary artery in an attempt to eliminate an external conduit. The combination of double switch procedures was as follows: Mustard and external conduit repair in 10 patients, Mustard and RV outflow reconstruction in 1, Senning and external conduit repair in 4, Mustard and arterial switch operation in 1, and Senning with arterial switch in 2 patients.
Atrial switch procedures
The Mustard procedure was performed through a vertical incision in the right atrium, and an additional transverse incision was made toward the confluence of the right pulmonary veins to create a T-shaped opening. After liberal atrial septectomy, a systemic venous channel was created with a glutaraldehyde-treated equine pericardial baffle (Xenomedica AG, Luzern, Switzerland). The ostium of the coronary sinus was always incorporated in the systemic venous channel, because the anterior conduction system was the rule in AV discordance. In patients with bilateral superior venae cavae, the coronary sinus was cut back deep into the left atrium and its cranial wall was used as a pedicled flap to partially create a ceiling of the pulmonary venous channel in patients 2, 9, and 11. Similarly, in patient 3 partial pulmonary venous return into the right superior vena cava was corrected by diverting the inferior vena cava and persistent left superior vena cava into the anatomic left atrium by a partial Mustard baffle, in which the ostium of the right superior vena cava into the anatomic right atrium was kept and the right superior vena cava distal to the anomalous pulmonary venous connection was ligated. In all Mustard cases, the functional left atrium was deliberately enlarged with Xenomedica membrane or an autologous pericardial patch down to the confluence of the right pulmonary veins.
In patients having the Senning operation, a standard Senning procedure was performed in patients 1, 4, 8, and 12, and cutback of the coronary sinus was added in patients 5 and 7, who had a persistent contralateral superior vena cava.
Switch procedures at VA level
An arterial switch operation, done by the French maneuver in the presence of a normal pulmonary valve, was performed in patients 1, 2, and 7. Mirror image of the normal coronary pattern was seen in all cases and round coronary buttons were implanted into punched-out holes in the facing wall of the pulmonary artery above the level of the sinus of Valsalva. The VSD was closed through the right atrial approach. Pledget-supported mattress sutures were used exclusively in all patients. Those sutures were placed on the anatomic RV side along the cranial and anterior margin of the defect and on the margin of LV aspect of the ventricular septum along its posterior margin.
In external conduit repair, ventricular rerouting was performed with a double-layered Xenomedica membrane. The intraventricular conduit was also fixed in place with pledget-supported mattress sutures placed exclusively on the RV side of the ventricular septum and then onto the aortic valve ring mainly with a running suture. Along the posterior margin of the defect, sutures were placed through the tricuspid valve ring and then directly on the margin of the VSD, disregarding the possibility of the posterior conduction system regardless of the visceral situs and of the VA connection. An extracardiac conduit was placed from the left to the ascending aorta in situs solitus and from the right to the ascending aorta in situs inversus.
A VSD extending from the membranous septum to the aortic valve ring without a recognizable infundibular septum (total conus defect in our classification) was the best indication for rerouting, and this favorable type of VSD was seen in 3 of 15 patients undergoing intraventricular rerouting (patients 3, 9, and 16). In the remaining 11 patients, a VSD was located in the membranous portion of the septum with various degrees of infundibular extension, as seen in patients with ordinary tetralogy of Fallot. In patients 13 and 16, the defect was located in the middle of the infundibular septum (mid-conus defect according to our classification, or infundibular muscular defect). Provided the anterior conduction system was present in all cases, its protrusion on the RV side could be carved safely. The outlet portion of the septum generally was composed of infundibular septum and the original ventricular septum, in which the former covered the cranial aspect of VSD on the RV side. Because the infundibular septum should not contain conduction system, its resection should be done safely. It was our practice to evaluate the anatomic composition of the VSD in the LV aspect through a right atrial approach. Along the anterior margin of the VSD, the lower margin of the infundibular septum could occasionally be recognized as a second margin on the opposite side of the septum restricting the VSD. This portion of the infundibular septum could be resected. Also, a wedge resection of the ventricular septum proper at the posterocaudal corner of the VSD was naturally possible. In patients with double-outlet RV with AV discordance with or without pulmonary atresia, resection of the infundibular septum also could be performed after the structure on the LV surface was analyzed. Enlargement of the VSD was necessary in 4 patients (patients 4, 6, 11, and 15) and carving of the infundibular septum was performed in 2 (patients 13 and 14).
An extracardiac conduit bearing three cusps was hand made with Xenomedica membrane on the operating table. 19 Diameters of the conduit ranged from 20 to 26 mm. In patient 18, the distal end of the pulmonary artery was brought down to the cranial margin of the right ventriculotomy to the left of the aorta and a direct anastomosis was performed to create an autologous posterior wall of the outflow tract, which then was enlarged with an autologous pericardial patch bearing a monocusp.
Repair of AV valve
Mitral regurgitation resulting from a cleft anterior leaflet was repaired by suture closure and annuloplasty in patients 2, 3, and 4. Mitral valve replacement with a 23 mm St. Jude Medical valve (St. Jude Medical, Inc., St. Paul, Minn.) had to be done in patient 2 after unsuccessful valve repair.
Mild to severe systemic tricuspid regurgitation was seen in 9 patients before the operation, and Reed's tricuspid annuloplasty was performed in 5 patients at the commissure between the septal and posterior leaflets.
Other concomitant procedures
An accessory pathway was confirmed between the left atrium and anatomic RV by epicardial mapping, and Sealy's operation combined with cryoablation for type A Wolff-Parkinson-White syndrome was performed in patients 5 and 6. In patient 15, repeated episodes of paroxysmal supraventricular tachycardia were controlled by Sealy's operation and cryoablation, and prosthetic aortic valve replacement with a 19 mm St. Jude Medical valve was also performed. In patient 1, a pacemaker was implanted for congenital AV block.
RESULTS
All but 2 patients survived the operation, and the hospital mortality rate was 11%. One (patient 17) died of pulmonary hypertension and septicemia 33 days after the operation and another (patient 2) died of low cardiac output because of massive regurgitation of the neoaortic valve 3 days after the operation. In patient 17, who had associated pulmonary atresia, preoperative measurement of pulmonary vascular resistance index was erroneous. Pulmonary mean arterial pressure measured by insertion of a catheter through a Blalock-Taussig shunt yielded a low pressure of 8 mm Hg and the resultant pulmonary vascular resistance index was reported to be 1.3 Wood units. However, postoperative catheterization disclosed a prohibitive pulmonary vascular resistance index of 9.3 Wood units. In retrospect, preoperative catheterization data were reevaluated. The left pulmonary venous wedge pressure was found to be elevated (33 mm Hg) and pulmonary vascular resistance index was corrected to 9.4 Wood units, a figure that should have contraindicated total correction.
Follow-up in 16 survivors ranged from 4 to 46 months (mean 22.9 ± 10.4 months). Currently 14 are in New York Heart Association functional class I and 2 are in class II. Reoperation for repair of superior vena caval obstruction 1 month after the operation and closure of residual shunts at both the atrial and ventricular levels 31 months later were performed in patients 10 and 4, respectively. None had permanent surgical AV block after the double switch operation. Regarding residual regurgitation of AV valves, postoperative tricuspid regurgitation was grade 1/4 in 3 patients by cineangiogram and was judged trivial by Doppler echocardiogram in the other 7 patients. Systemic mitral regurgitation was grade 1/4 in 4 patients on cineangiogram, and an additional 4 patients were found to have trivial regurgitation by Doppler echocardiogram.
Postoperative parameters
After the double switch operation, the cardiothoracic ratio increased from 50.9% ± 6.4% to 58.9% ± 4.7% (p = 0.0001). Anatomic RV end-diastolic volume showed a significant reduction from 122.2% ± 46.3% of normal to 78.4% ± 24.3% because of unloading of the ventricle and diversion of its outflow tract into the systemic ventricular outlet by intraventricular rerouting after the operation (p = 0.0002), and RV ejection fraction remained unchanged (56% ± 8% to 56% ± 6%). Postoperatively, systolic pressure in the RV showed a marked decrease to 44.7 ± 17.4 mm Hg and RV/LV systolic pressure ratio decreased to 0.49 ± 0.2. The anatomic LV end-diastolic volume and LV ejection fraction remained unchanged before and after operation: end-diastolic volume 134.6% ± 50.0% of normal to 127.3% ± 32.8% (p = 0.4859) and ejection fraction from 61% ± 8% to 56% ± 8% (p = 0.0606). Cardiac index averaged 3.1 ± 0.5 L/m2 after the operation, ranging from 2.0 to 4.4 L/m2. Changes in LV ejection fraction before and after the double switch operation showed an increase in 7 patients and a decrease in 8 patients (Fig. 1). In 3 patients (patients 6, 11, and 12), medium-term follow-up catheterization was performed 1 to 1 years after the operation. In patient 6, LV ejection fraction increased from 0.42 at 1 month after operation to 0.50 at 1 year, and cardiac index also increased from 2.6 to 3.4 L/min per square meter. In the other 2 patients, exercise testing by supine bicycle ergometer was performed during catheterization. Marked increases in cardiac output compared with the resting state were observed. Cardiac index increased from 3.3 and 3.0 L/min per square meter at rest to 5.5 and 4.6 L/min per square meter after exercise in patients 11 and 12, respectively.
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Arterial switch operation
In 2 patients who survived the combined Senning and arterial switch operation (patients 1 and 7), RV end-diastolic volume showed a marked decrease from 213% to 115% of normal in patient 1 and from 241% to 125% of normal in patient 7 because of the unloading effect after the operation. Postoperative systemic LV function revealed excellent results. LV end-diastolic volume decreased from 189% to 154% of normal in patient 1 and 267% to 194% of normal in patient 7. Postoperative LV ejection fractions were 0.53 and 0.55 and cardiac indexes were 3.2 and 3.1 in patients 1 and 7, respectively.
DISCUSSION
Long-term fate of the anatomic RV as a systemic ventricle still remains controversial in patients with AV discordant anomalies. 1-6 Systemic RV failure usually associated with tricuspid regurgitation after the Mustard or Senning procedure in complete transposition of the great arteries was experienced with increasing frequency, 7-14 and function of the failing RV can be normalized as the pulmonary ventricle by conversion from an atrial switch to an arterial switch operation. Also, our clinical experience in conversion from atrial switch to arterial switch after systemic RV failure in 6 patients with transposition, 5 of whom survived and were doing well, showed that the systemic LV greatly improved the quality of life in these patients. In view of the facts that systemic RV function rapidly deteriorates in association with tricuspid regurgitation and that tricuspid regurgitation of the systemic RV is one of the major risk factors in conventional surgical correction of associated anomalies in atrioventricular discordance, 2,6 the double switch operation or anatomic correction carries the best hope for longevity.
Anatomic correction in atrioventricular discordance as a combined atrial switch and Rastelli repair has been reported by Ilbawi and associates 20 in 1990 and by Di Donato and colleagues 21 in 1992. Combined arterial switch and atrial repair as an anatomic repair for AV discordance has never been reported to our best knowledge except for our preliminary report. 22 In our series, the first double switch operation, consisting of a Senning and arterial switch operation, was performed on a 2-year-old boy with atrioventricular discordance associated with grade 3 tricuspid regurgitation on June 12, 1989, with success; a combined Mustard and Rastelli-type repair was then performed on May 25, 1990, also with success (Figs. 4 and 5). In 1992, direct anastomosis between the distal pulmonary artery and cranial margin of the right ventriculotomy was also successfully performed, and this modification, similar to REV (réparation à l'etage ventriculaire) in TGA reported by Lecompte and coworkers, 23 would further reduce long-term morbidity and need for reoperation by eliminating a tubular prosthetic material in the outflow reconstruction. Two types of anatomic repair, combined arterial switch and the Senning procedure and combined atrial switch and direct anastomosis between the RV and the distal pulmonary artery, seemed to be new combinations performed for atrioventricular discordance.
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Arterial switch operation
Combined atrial and arterial switch operation is preferable to conventional repair in patients with tricuspid regurgitation, because conversion of the systemic RV to a pulmonic ventricle even without direct surgical intervention on the tricuspid valve provides an excellent solution to a hitherto complicated problem. In 2 surviving patients after the combined Senning and arterial switch operation (patients 1 and 7), RV end-diastolic volume showed a marked decrease because of the unloading effect after anatomic correction, and new systemic LV function also was excellent. LV end-diastolic volume decreased from 189% to 154% of normal in patient 1 and from 267% to 194% of normal in patient 7. Postoperative LV ejection fraction was 0.53 and 0.55 and cardiac index was 3.2 and 3.1 in patients 1 and 7, respectively. However, massive neoaortic regurgitation seen in patient 2 with subvalvular pulmonary stenosis contraindicates conversion of the untrained low-pressure pulmonic valve into a neoaortic valve. Because combination of the arterial switch and Senning procedures revealed excellent systemic LV function after the operation, universal application of this type of anatomic repair should be considered in cases of atrioventricular discordance associated with VSD and pulmonary hypertension in the future.
Tricuspid regurgitation
Systemic tricuspid regurgitation was considered to be a major problem in the surgical treatment of patients with atrioventricular discordance, and Ebstein's anomaly of the systemic AV valve was not amenable to conventional valvuloplasty. 2,24-26 It is also worth mentioning that postoperative tricuspid regurgitation was clinically insignificant in all patients including one with Ebstein-like anomaly despite simple application of tricuspid annuloplasty in 5 of 9 cases with preoperative tricuspid regurgitation. Significant reduction in both the end-diastolic volume and peak systolic pressure after the double switch operation made the incompetent tricuspid valve competent. Although longer follow-up is warranted, the double switch operation should be best indicated for atrioventricular discordance especially in the presence of systemic tricuspid regurgitation or in patients with poor systemic RV function.
VSD
Location and size of the VSD was important in this type of repair. In isolated VSD in atrioventricular discordance, we had often seen the typical perimembranous VSD with a long infundibular septum. However, in cyanotic patients with atrioventricular discordance, some degree of infundibular extension of the VSD seemed to be the rule, partly by deviation of the infundibular septum toward the pulmonary artery in patients with pulmonic stenosis or by maldevelopment of the infundibulum in patients with double-outlet RV and pulmonary atresia. 27 This infundibular extension of varying degree is not necessarily unique to Asian populations and is also clearly shown on the angiogram reported by Ilbawi and his associates. 20 In cyanotic patients, ejection by the anatomic LV into left-sided anteriorly originating aorta, predisposing straight alignment of the ventricle to the aorta, resulted in dextrocardia or mesocardia in its cardiac position in {SLL} and in levocardia or mesocardia in {IDD}.
Conduction system and postoperative arrhythmia
General agreement has been reached concerning the anterior conduction system in {SLL}. 28 On the other hand, the posterior conduction system has been reported in atrioventricular discordance in situs inversus. 29,30 In our series of atrioventricular discordance including conventional Rastelli repair, there were 11 cases of {IDD} type, in which the VSD was either closed or rerouted. We merely preserved the anterior conduction system and disregarded the posterior counterpart, but sinus rhythm could be maintained in all cases after the operation. Because there was no difference in AV septal alignment either in {SLL} or in {IDD}, an anatomic explanation seemed lacking to support the different conduction pattern in all cases of {IDD}. However, there was a possibility of the dual pathway forming a sling in atrioventricular discordance. The exact prevalence of posterior conduction in a large series of patients, especially with a good AV septal alignment, should be studied in detail. Regarding patients with Wolff-Parkinson-White syndrome or with preoperative paroxysmal supraventricular tachycardia, recurrence of tachyarrythmia was seen in patient 7 and was controlled with antiarrhythmic medication. In the remaining 2 patients, arrhythmia surgery combined with cryoablation successfully prevented recurrence.
In summary, although the double switch operation is a time-consuming procedure and longer follow-up is warranted, it is best indicated for patients with atrioventricular discordance, especially in the presence of systemic tricuspid regurgitation or in patients with poor systemic RV function. Preoperative tricuspid regurgitation improved spontaneously without surgical intervention on the valve, and mere unloading of the anatomic RV was sufficient to control the regurgitation in our series. Postoperative ventricular function can be favorably compared with that of the conventional Rastelli procedure in complete transposition of the great arteries. Combined atrial switch and arterial switch procedures were performed in 3 patients, and 2 surviving patients are currently doing well in New York Heart Association functional class I. This combination anatomic repair should be considered for patients with atrioventricular discordance associated with VSD and pulmonary hypertension. Two types of anatomic repair, combined arterial switch and Senning procedure and combined atrial switch and direct anastomosis between the RV and the distal pulmonary artery, are new combinations reported for the treatment of patients with atrioventricular discordance.
Appendix: DISCUSSION
Mr. Jaroslav F. Stark(London, England).
We have recently reviewed our experience at Great Ormond Street with 90 consecutive patients with AV discordance operated on between 1974 and 1990. Two incremental risk factors for death had high significance—anatomically abnormal tricuspid valve and tricuspid valve incompetence. These observations would certainly support your idea that the so-called double switch operation is preferable to the standard repair. Also, as you are closing the VSD from the RV side, I presume the risk of complete heart block would also be reduced. You have reported one pacemaker implantation. Was this the result of surgical heart block or was the patient already in complete heart block before the operation?
Mr. Marc R. de Leval (London, England).
Some years ago I looked at a number of hearts with AV discordance to define the relationship between the aortic valve and the VSD. My intention was to try to decide preoperatively which VSDs could be closed through the aorta. I noticed a great anatomic variety of relationships. Quite a few hearts had a large infundibular septum between the aorta and the VSD. Furthermore, in a number of specimens there was a dysplastic tricuspid valve with chordal attachments on the margin of the VSD and/or the infundibular septum. Sometimes the defect was partially obstructed by the tricuspid valve.
My question is this: Do you think that you can do a Rastelli procedure in all patients or do you think that some of them should be excluded? Furthermore, if you want to enlarge a VSD, where do you make the incision, knowing that the conduction system runs along the anterosuperior aspect of the defect?
Dr. Serafin Y. DeLeon (Maywood, Ill.).
This just goes to show that surgeons really think alike. When we reported our experience in 1990, we did not call it a new approach because of the possibility that somebody had done it before.
Your experience is nice. Combining the arterial switch and Mustard procedure is the ideal thing to do. In certain cases, however, a Rastelli procedure using a conduit is needed. In these cases, instead of using a conduit, we have uprooted the main pulmonary artery and valve, swinging it to the RV. We do this with the hope that these patients will never require further operations. Conduits will be outgrown or degenerate and will have to be replaced.
Dr. Imai.
First, I would like to thank Mr. Stark for his kind comments. Regarding heart block, one patient with congenital heart block required pacemaker implantation. However, we had no case in this series in which surgical block was induced. As far as tricuspid regurgitation is concerned, I completely agree with your view. We did have the same dismal follow-up study in patients with the systemic tricuspid regurgitation, whether it was repaired by the valvuloplasty or valve replacement. That is one reason why we started this kind of approach in this subset.
In response to Mr. de Leval, enlargement of the VSD is sometimes difficult. And the types of VSDs found in the AV discordant anomalies varies a great deal. However, if you select patients with a VSD and pulmonic stenosis or atresia, most of the patients do have a so-called malaligned-type VSD, in that the length of the infundibular septum is not really long. Then, as you know, the ventricular septum is composed of ventricular septum proper overlapped by the infundibular septum on its RV side. As long as you are resecting the infundibular septum, not the ventricular septum proper, it is safe. In patients with the normal visceroatrial situs, the posterior margin of the VSD can be enlarged at your will, provided anterior conduction is the rule.
Dr. DeLeon, I quite agree with your view. It is our custom now, so as to avoid prosthetic material while doing a conventional Rastelli repair in the complete transposition of the great arteries, to pull the distal part of the pulmonary artery directly on the RV, as suggested by Lecompte. In the last patient of our series, usage of an external conduit was avoided by direct anastomosis.
Footnotes
From the Department of Pediatric Cardiovascular Surgerya and Pediatric Cardiology,b The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan. 
Read at the Seventy-third Annual Meeting of The American Association for Thoracic Surgery, Chicago, Ill., April 25-28, 1993.
References
This article has been cited by other articles:
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M. Koh, T. Yagihara, H. Uemura, K. Kagisaki, I. Hagino, T. Ishizaka, and S. Kitamura Intermediate Results of the Double-Switch Operations for Atrioventricular Discordance Ann. Thorac. Surg., February 1, 2006; 81(2): 671 - 677. [Abstract] [Full Text] [PDF]
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V. Hraska, B. W. Duncan, J. E. Mayer Jr, M. Freed, P. J. del Nido, and R. A. Jonas Long-term outcome of surgically treated patients with corrected transposition of the great arteries J. Thorac. Cardiovasc. Surg., January 1, 2005; 129(1): 182 - 191. [Abstract] [Full Text] [PDF]
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 M. Koh, T. Yagihara, H. Uemura, K. Kagisaki, and S. Kitamura Functional biventricular repair using left ventricle-pulmonary artery conduit in patients with discordant atrioventricular connections and pulmonary outflow tract obstruction--does conduit obstruction maintain tricuspid valve function? Eur. J. Cardiothorac. Surg., October 1, 2004; 26(4): 767 - 772. [Abstract] [Full Text] [PDF]
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Y. Isomatsu, T. Shin'oka, M. Aoki, M. Terada, T. Takeuchi, S. Hoshino, Y. Takanashi, Y. Imai, and H. Kurosawa Establishing right ventricle-pulmonary artery continuity by autologous tissue: an alternative approach for prosthetic conduit repair Ann. Thorac. Surg., July 1, 2004; 78(1): 173 - 180. [Abstract] [Full Text] [PDF]
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S. M. Langley, D. S. Winlaw, O. Stumper, R. Dhillon, J. V. de Giovanni, J. G. Wright, P. Miller, B. Sethia, D. J. Barron, and W. J. Brawn Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries J. Thorac. Cardiovasc. Surg., June 1, 2003; 125(6): 1229 - 1241. [Abstract] [Full Text] [PDF]
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M. Ando, B. W. Duncan, and R. B.B. Mee Anatomic correction for corrected transposition after pulmonary unifocalization Ann. Thorac. Surg., March 1, 2003; 75(3): 1012 - 1014. [Abstract] [Full Text] [PDF]
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M. N. Ilbawi, C. B. Ocampo, B. S. Allen, M. J. Barth, D. A. Roberson, P. Chiemmongkoltip, and R. A. Arcilla Intermediate results of the anatomic repair for congenitally corrected transposition Ann. Thorac. Surg., February 1, 2002; 73(2): 594 - 600. [Abstract] [Full Text] [PDF]
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 T. P. Graham Jr., Y. D. Bernard, B. G. Mellen, D. Celermajer, H. Baumgartner, F. Cetta, H. M. Connolly, W. R. Davidson, M. Dellborg, E. Foster, et al. Long-term outcome in congenitally corrected transposition of the great arteries: A multi-institutional study J. Am. Coll. Cardiol., July 1, 2000; 36(1): 255 - 261. [Abstract] [Full Text] [PDF]
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M. Imamura, J. J. Drummond-Webb, D. J. Murphy Jr, L. R. Prieto, L. A. Latson, S. D. Flamm, and R. B.B. Mee Results of the double switch operation in the current era Ann. Thorac. Surg., July 1, 2000; 70(1): 100 - 105. [Abstract] [Full Text] [PDF]
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T. Millane, E. J. Bernard, E. Jaeggi, R. B. Howman-Giles, R. F. Uren, T. B. Cartmill, R. E. Hawker, and D. S. Celermajer Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries J. Am. Coll. Cardiol., May 1, 2000; 35(6): 1661 - 1668. [Abstract] [Full Text] [PDF]
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M. L. Jacobs, M. J. Buckley, and R. L. Liberthson Combined rastelli and atrial switch procedure: a 10-year follow-up Ann. Thorac. Surg., August 1, 1999; 68(2): 570 - 571. [Abstract] [Full Text] [PDF]
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T. Yeh Jr, M. S. Connelly, J. G. Coles, G. D. Webb, P. R. McLaughlin, R. M. Freedom, P. B. Cerrito, and W. G. Williams ATRIOVENTRICULAR DISCORDANCE: RESULTS OF REPAIR IN 127 PATIENTS J. Thorac. Cardiovasc. Surg., June 1, 1999; 117(6): 1190 - 1211. [Abstract] [Full Text] [PDF]
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H. M. Connolly, M. Grogan, and C. A. Warnes Pregnancy among women with congenitally corrected transposition of great arteries J. Am. Coll. Cardiol., May 1, 1999; 33(6): 1692 - 1695. [Abstract] |
